A normal lymphatic system consists of blind-end vessels which collect the fluid that bathes and nourish the tissues. In a normal system, this fluid, called lymph, is derived from the arterial side of capillaries, and is returned to the circulation via veins near the neck. Nearly 90% of the water-component in our blood is consistently filtered through the lymphatic system while the remaining 10% is found in the tissues. The purpose of the lymphatic system is to help the body to maintain fluid balance while filtering out waste products. White blood cells and other immune cells congregate in lymph nodes in various parts of the body (notably the armpit and groin region). They help to destroy bacteria, cancerous cells or other wastes that make their way into this lymph fluid.

Lymphedema is a swelling in the soft tissues of the limbs, or less often the trunk, caused by the buildup of lymph fluid. This can occur in two ways:

primary lymphedema, a rare genetic condition which children may be born with, may become evident in their teens (lymphedema praecox) or in adulthood (lymphedema tarda) or

secondary lymphedema, which is due to damage to the lymphatic system as a result of trauma, parasites (in developing countries) surgery or radiation therapy. In contrast to edema, a condition where the lymphatic system is intact but overwhelmed, lymphedema results from mechanical insufficiency in which both water and proteins accumulate. Therefore, the lymphatic system cannot remove even normal amounts of lymph from the tissues. (1) When the lymphatic system is damaged beyond repair, persistent lymphedema becomes a chronic condition.

Over 2 million women in the United States are breast cancer survivors. The American Cancer Society estimates that 10-15% of these women (200,000-400,000)(2) , although others have proposed an even higher estimate of 30-40% (600,000 to 800,000) women will develop lymphedema over their lifetime. (3) A 1998 review article of 7 large reports published since 1990 report an incidence of lymphedema in 6-30% of breast cancer survivors treated in 5 Western countries (3). Approximately 5% of breast cancer survivors noted lymphedema after their first year of treatment.(3) A study that questioned 263 women 20 years after their treatment showed that 49% had experienced at least mild lymphedema, and that 11% experienced severe cases. Seventy seven percent of the women who had lymphedema reported early onset swelling (within 3 years of diagnosis) and the other had late onset of symptoms at a rate of 1% per year. This late onset lymphedema correlated with two epidemiologic factors: a history of infection or injury, and weight gain since treatment. (4) (This will be further discussed later)

In part because lymphedema may develop weeks, months or years following treatment for breast cancer, it is difficult to accurately determine the exact causes of lymphedema. Long-term studies of women with differing life styles, treatments and backgrounds have not shown a relationship between age, obstructive drainage, number of lymph nodes to which cancer had spread, or weight at diagnosis(4). They have, however, identified relationships between the following and the onset of lymphedema:

Surgery: Axillary dissection (the surgical removal of lymph nodes under the arm) or the destruction of lymph nodes. A large (1278 patient) study showed that 15.9% of women undergoing axillary dissection and radiation therapy developed lymphedema and that the risk correlated with number of lymph nodes removed. (5) Other studies noted that there was no difference in the rate of lymphedema between women who had undergone modified radical versus radical mastectomy, and breast conserving surgeries such as lumpectomy, axillary dissection and radiation therapy.(4)

Radiation therapy: This may cause damage or scarring to lymph nodes or lymph vessels leading to lymphedema in approximately 30% of women who have undergone radiation therapy. Several studies (4,6) also noted a significant increase in lymphedema for women who have undergone radiation therapy to either the axilla, or from radiation scatter to the breast or chest wall.

The foremost reasons why women developed late-onset lymphedma (3 or more years following therapy) are weight gain following cancer treatment, infection and injury. It is therefore advisable for women who have undergone treatment for breast cancer to be aware of the following:

Infection (cellulitis: bacterial infection of the skin or lymphangitis: infection of the lymphatic vessels or system) can lead to an increase in blood flow, and thus an increase in lymph build-up in the affected area. Lymphangitis may also cause obstruction of lymphatic vessels and consequent lymphedema due to lymph back-up. Oral antibiotics may be prescribed by a physician if an infection does occur.

Weight gain following cancer therapy should be avoided as much as possible as it has been shown to increase susceptibility to lymphedema. Although patients who were overweight at the time of their diagnosis had a higher incidence of lymphedema than women of optimal weight, weight gain in post-treatment years were shown to be a stronger predictor of lymphedema development (4). A nutritionist may be consulted to encourage a balanced low fat, high fiber diet along with some form of exercise to help maintain an ideal weight .

Injury: It is important to avoid wounds in the arm on the same side of a mastectomy or lymph node dissection. Even minor scrapes or sterile needle pricks should be avoided as they may trigger the onset of lymphedema. Any wounds should be cleaned properly with antibiotic soap. Prophylactic (to avoid the onset of) oral antibiotics may be prescribed by a physician to reduce the chance of infection.

Other incidences that cause blood to rush to the area, such as overuse of the affected limb, sudden changes in temperature (hot tub, sauna), trauma and vigorous massages are also implicated in initiating or aggravating existing lymphedema..

Airplane flights: even for people without lymphedema the low pressure may cause swelling. This swelling is accentuated in women susceptible for lymphedema or with a mild case.

Macrophages, a large immune cell that engulfs bacteria and other foreign substances, are not as efficient in the lymph fluid as in fluid bathing tissues

A condition termed dermatolymphangioadenitis (DLA), includes all types of inflammatory changes to the skin including erysipelas (streptococcal infection of the skin), cellulitis (bacterial soft tissue infection) and lymphangitis (infection of the lymphatic system) attest to the increase frequency of infection. They are the most common complication of lymphedema affecting over 50% (and significantly more in tropical regions) of those with lymphedema. Symptoms of infection may include: (7)

If these symptoms occur, it is best to see a doctor who can prescribe oral or intravenous antibiotics depending on the severity. Rest, elevating the affected limb, increasing fluid intake and avoiding direct sunlight are also recommended. (8)

In early stage of lymphedema
Wounds from daily activities, such as gardening, kitchen cuts, and pet scratches are the culprit of infection. It is important to wear gloves while doing housework, to avoid cutting your cuticles, to wash frequently with mild antibiotic soaps, to dry skin gently but thoroughly following bathing and to keep area moist with lotion. Any minor wounds may be cleaned with either diluted hydrogen peroxide (50% water, 50% hydrogen peroxide) or antibiotic soap. Antibiotic cream, such as Neosporin or Silvadene should then be applied and the wound protected with gauze. Any infection that occurs should be brought to a physician's attention and treated with systemic antibiotics (prescription).

In more severe cases of lymphedema
The skin becomes fibrotic and develops cracks. These are portals for bacteria which may cause cellulitis. Furthermore, skin folds may harbor fungal infections or skin breakdown. Again, it is very important to be careful about injuring skin. Areas between fingers or under skin folds should be kept dry to avoid fungal growth, and the limb should be checked frequently for any changes in color or temperature.

Very advanced lymphedema
This is generally associated with leakage of lymph from the skin. This can be due to maceration (breakdown) of skin or from papilloma (blister-like bubbles) that open and lead to chronic oozing and increased risk of infection.

In order to adequately control an infection, it is vital to clean and dress the area. This includes:

Dress the wound. This will allow for a moist environment without maceration, and speed healing. It is important to choose a dressing that fills the hollow space of a wound and absorbs the exudates. These include transparent films, foam alginate (an absorptive dressing made from seaweed), hydrocolloid, hydrogel (synthetic colloid that can absorb water) for minimal to moderate absorption. Maximum absorption is best done through the use of dry gauze and infant diapers wrapping the wound.

A compress wrap around the affected limb provides thermal insulation, protection against trauma, and increases oxygen tension in the tissues, thereby making it a critical part of healing wound in lymphedema patients. It should be noted that this should be discontinued if cellulitis develops.

Other factors which may reduce the speed of wound healing include nutrition, drugs (chemotherapy, steroids and aspirin), smoking, diabetes and atherosclerosis. A health professional may help in keeping diabetes at bay while improving nutrition Nutrition Module, finding alternatives to certain drugs, and encouraging smoking cessation.

Unfortunately, there is no consensus regarding standard treatments for lymphedema. There has been limited controlled research on the efficacy of individual treatments, let alone their combination. Often, treatment options are selected individually based on past medical history, amount of edema, lifestyle and insurance/financial situation. The most commonly suggested treatments involves decongestive physiotherapy, which include the use of compression devices (compression bandages/ sequential lymphatic pumps) and Manuel Lymphatic Drainage (a gentle massage). Drug therapy is also described, although somewhat controversial in effectiveness.

To determine whether a treatment is working for you, measure the arm before and after you start. This may be done 5 inches above and below the elbow, at the wrist and across the knuckles. Take the same measurements of the other arm and use them as a comparison. Keep a chart with the dates, treatment used and measurements. If the measurements go down or at least stay the same, the program is effective.

Compression devices: Since the elastic fibers in the skin may be damaged by lymphedema, (9) compression devices may help to prevent stretching the skin, protect the limb from trauma and reduce swelling. As muscles contract against an inelastic barrier, the efficiency of the muscle pump promotes re-absorption at the venous end of capillaries while decreasing the amount of fluid filtered out at the arterial end into the lymphatic system. Pressure of the garment should be as high as the patient can tolerate to ensure the maximum benefit.(9)

Proper fitting of a compression device is essential. A nurse or other healthcare professional may help find the best size and material for you, and teach you how to properly wrap your arm. There are several devices available:

Bandages Wrapping limb with a low stretch bandage and padding or foam allows for a low resting pressure and high working pressure which encourages lymph flow. (These bandages can be purchased from the same vendors that sell the garments.) Because each bandage is wrapped to custom fit, it can help to reshape the arm. Unfortunately, it is also difficult to apply. Professionals are encouraged to teach patients how to properly apply the bandage to avoid constriction. Rather than reducing lymphedema, constriction may in fact increase limb swelling. Wrapping should be done in spiral direction not circular turns. The circular turns can too easily become a tourniquet. Numbness in the hand is usually the warning sign that the bandage is too tight. Coldness or change in the color of the fingers is danger sign and bandage should be removed immediately.

Static Compression Devices These may be used alone or in conjunction with a compression bandage. A study, however, has shown a significant decrease in limb volume when both a compression bandage and compression garment are used together as compared to a garment alone. (6) Compression garments are made of foam covered by an inelastic outer casing and are generally fastened with Velcro. This allows them the variability of being flexible during exercise to rigid at rest. The down-point is that static compression devices are generally costly and are not covered by most insurance companies.

Lymphedema Pumps These were used in the past, but their efficacy is now being debated. They are generally rented by the month and must be used for 2-4 hours per day for 2 weeks. Most vendors will teach you how to use the pump: A sleeve, which comes with pump, should be pulled up to the arm pit. The arm is then elevated as high as possible and the machine set to 10 points below diastolic pressure (with a blood pressure of 120/80, the diastolic pressure is 80). After pumping and then wrapping arm with a bandage for 2 weeks, a compression device may be worn to keep the limb a specific size

Therapeutic Massage or Manual Lymph Drainage:
The term massage is a misnomer for this very gentle stimulation of lymph flow. Unlike traditional massage which affects the deeper muscles and tissues, and may actually cause an increase of lymphedema, this form affects the lymphatic vessels just beneath the skin. Also called manual lymph drainage (MLD), it consists of slight rhythmic pressure which improves the activity of intact lymph vessels by mechanical stretch on the walls of lymph collectors, and thus a better filling of lymph vessels. A MLD professional or nurse may be helpful in teaching proper self-technique so that drainage may be done in the comfort of home.

In the presence of severe edema, you can try to move fluid to the unaffected arm pit (axilla). Using stationary circles move fluid across the breast bone to the unaffected side. You can also move fluid across the back to the unaffected arm pit. In addition you can try to move fluid through the abdomen to lower lymph drainage areas. Abdominal drainage is done in four quadrants: lower left quadrant, lower right quadrant, upper right quadrant, and upper left quadrant. Pressure is applied towards the spine in direction of the navel. Deep abdominal drainage is done in combination with breathing. Pressure is applied straight down with exhalation. Apply resistance to inhalation, release at the top of the breath, and move to the next position:

a. in center above navel
b. under ribs-left side
c. above hip bone-left side
d. under ribs-left side
e. in center above navel
f. under ribs-right side
g. above hip bone-right side
h. under ribs-right side
i. in center above navel

There are many other maneuvers that can be taught by MLD trained personnel.

The suggested intensive treatment of superficial lymphedema includes four parts, although it is best to check with a doctor for personal regimens. These are generally suggested to be done before bedtime and include:

Manual lymph drainage to remove as much stagnant fluid in the arm and to stimulate the flow of lymph back into the venous system

Skin care, including the use of oil or cream following MLD to keep the skin moist and to prevent cracking

Compression bandaging of the affected limb. This will prevent the flow of lymph fluid back into the arm during sleep and is best kept for a 24 hour period following MLD.

Mild exercise to promote the action of muscle pumps which may stimulate the movement of lymph fluid. Exercise Module

Benzopyrones These have been shown to stimulate macrophage (immune cell) activity and to promote the breakdown of proteins in the lymph fluid. With a decrease in proteins in lymph comes a decrease in the water/fluid which accumulates and thus a decrease in lymphedema. The effectiveness of Benzopyrones is still being evaluated

Diuretics These promote excess fluid in the body to be excreted. Although they may help to decrease lymphedema in the short term, diuretics are not recommended over the long-term.